An Infant with Persistent Respiratory Failure Associated with Refractory Pulmonary Hypertension: Pulmonary Interstitial Glycogenosis

IF 0.3 Q4 PEDIATRICS
Gianfranco Tomarelli, A. Donoso, Francisca Andrades, S. Montes
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引用次数: 1

Abstract

Pulmonary interstitial glycogenosis (PIG) is a disease of unknown etiology. It is part of the interstitial lung diseases, corresponding to the compartment of the fetal pulmonary interstitium. It typically presents within the first week of life as refractory respiratory distress with tachypnea and persistent hypoxemia, and it is not associated with glycogen deposition in other organs. Usually, there is a clinical improvement and good prognosis after steroid therapy unless there are associated conditions such as congenital heart disease, pulmonary hypertension, or genetic disorders. We report a case diagnosed by lung biopsy at 4 months of age in a male preterm born, small for gestational age infant, who developed refractory hypoxemia and pulmonary hypertension with fatal outcome. There was no response to steroids and hydroxychloroquine. He was not candidate for extracorporeal membrane oxygenation. PIG should be considered in the differential diagnosis of persistent respiratory distress and hypoxemia despite standard treatment, even after the first month of life.
顽固性肺动脉高压伴持续性呼吸衰竭的婴儿:肺间质性糖原症
肺间质糖原增多症(PIG)是一种病因不明的疾病。它是肺间质性疾病的一部分,与胎儿肺间质的隔室相对应。它通常在生命的第一周内表现为顽固性呼吸窘迫,伴有呼吸急促和持续低氧血症,与其他器官的糖原沉积无关。通常,类固醇治疗后会有临床改善和良好的预后,除非有先天性心脏病、肺动脉高压或遗传性疾病等相关疾病。我们报告了一例在4个月大时通过肺活检诊断为男性早产、小于胎龄婴儿的病例,该婴儿出现难治性低氧血症和肺动脉高压,最终导致死亡。对类固醇和羟氯喹没有反应。他不是体外膜肺氧合的候选人。尽管进行了标准治疗,但在鉴别诊断持续性呼吸窘迫和低氧血症时,即使在生命的第一个月后,也应考虑PIG。
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CiteScore
0.50
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